Is there a segmental form of Hirschsprung’s disease in children?
DISCUSSIONS
Abstract
Introduction. In the classification of A.I. Lenyushkin, there is a mention of the segmental form of Hirschsprung’s disease, as well as in a number of publications and parts of previously proposed classifications. However, the presence of a segmental form of Hirschsprung’s disease contradicts existing data on human embryogenesis. Aim — to determine the reliability of the existence of a segmental form of Hirschsprung’s disease. Materials and methods. Since 1991, we have observed 1,199 children suffering from various types of chronic constipation. Of these, there were 297 patients with various forms of Hirschsprung’s disease in the group of children with Hirschsprung’s disease. In 18 (1.5%) patients, irrigography above the normal rectum revealed narrowed areas alternating with enlarged sections, suspicious of the presence of a segmental form of the disease. At the same time, all the children were over two years old. To clarify the diagnosis, a comprehensive examination was conducted in this group. Results. Ultrasound examination of the colon did not show a decrease in its diameter in the narrowed areas. During video defecoscopy, the normal opening of the anal canal was noted without the formation of a protrusion of the walls of the rectum and its complete emptying. During the kolodynamic study, all children had a normal rectoanal inhibitory reflex. The vector volume pattern in this group of patients was in the form of an “hourglass” of a rather symmetrical shape, which is also characteristic of functional constipation. In this group of patients, negative acetylcholinesterase activity was detected in all biopsies of the mucous membrane taken from the narrowed zone of the colon. During the control irrigography performed three months later, narrowed areas in the above-mentioned areas of the colon were not detected. Conclusion. In a group of 1,199 children who were treated for chronic constipation at various times, none of the children had a segmental form of Hirschsprung’s disease. This fact casts doubt on the existence of the above-mentioned form.
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